Pemphigus Vulgaris

Background

Pemphigus vulgaris (PV) is a condition which causes blistering
of the outer layer of the skin (epidermis) and mucosal membranes,
including the nose and throat. It is a very rare condition and
occurs most commonly in middle-aged or older people of all races
and ethnic groups. Because PV is very rare, diagnosis may be
difficult or delayed. It is one of the three main types of
pemphigus, the other two forms being pemphigus foliaceus and
pemphigus paraneoplastic.

Pemphigus foliaceus (PF)
In Pemphigus Foliaceus, blisters and sores do not occur in the
mouth. Crusted sores or fragile blisters usually first appear on
the face and scalp and later involve the chest and back. The
blisters are superficial and often itchy, but are not usually as
painful as PV. In PF, disfiguring skin lesions can occur, but the
mortality (death) rate from the disease is lower than in PV.

Paraneoplastic pemphigus (PNP)
PNP is potentially the most serious form of pemphigus. It occurs
most often in someone who has already been diagnosed with cancer. Fortunately, it
is also the least common.

Painful sores of the mouth, lips and oesophagus are almost
always present; and skin lesions of different types occur. In some
cases, the diagnosis of the disease will prompt doctors to search
for a hidden tumour. In some cases the tumour will be benign and
the disease will improve if the tumour is surgically removed. It is
important to know that this condition is rare and looks different
than the other forms of pemphigus. The antibodies in the blood in
somebody with PNP are also different from other forms of pemphigus
and the difference can be determined by laboratory tests.

Credits

Medical text written September 2002 written by Contact a Family.
Approved September 2002 by Professor M Black, Consultant
Dermatologist, St John's Institute of Dermatology, Guy's, King's
and St. Thomas' School of Medicine, London, UK. Last updated June
2010 by Dr Richard Groves, Consultant Dermatologist, St John's
Institute of Dermatology, Kings College London, London, UK.

What are the symptoms?

PV causes the skin to separate easily and peel easily. For many
individuals, PV usually begins with blistering in the mouth and
throat. This may be followed by blistering or erosions of the skin,
including the groin, underarm, face, scalp and chest areas. PV
lesions may cover extensive portions of the body. In some affected
people the lesions are relatively asymptomatic. However, in the
healing stage following treatment, the lesions often crust over but
they no longer itch or burn and leak fluid. In some people the skin
lesions may itch and burn continuously and rupture which may leave
red erosions of the skin surrounded by a crust and scaling.
Affected areas usually heal without scarring, unless the lesions
become infected. Blisters in the mouth may make it difficult to eat
and drink, leading to problems with weight loss and
dehydration.

What are the causes?

PV is one of a group of chronic, relapsing conditions in which
the immune system produces antibodies against specific proteins in
the skin and mucous membrane, leading to the inability of the skin
cells to bind together. It is thought that PV may be triggered by a
range of factors. A few cases of PV have occurred following
reactions to medications, including penicillamine and captopril.
Individuals are probably genetically predisposed to PV. Specific
information about a gene change(s) will not, however, indicate the
certainty with which an individual will become affected with PV in
the future.

How is it diagnosed?

Diagnosis of these diseases is made by skin biopsy (where a
small amount of skin is removed for study in a laboratory) and
immunopathology (analysis of body fliuds to detect if there are any
antibodies attaking the proteins in the skin and mucous
membranes).

How is it treated?

There is no cure available for PV but in most cases available
treatments are highly successful in reducing symptoms and
preventing complications. PV is often initially controlled with
high-dose steroids together with one of a number of so-called
'steroid sparing' immunosuppressive drugs. Response to medication
varies from individual to individual and, for some people, lesions
may not heal for extended periods of time. The main risk from PV is
infection and complications resulting from medication aimed at
suppressing the immune system. Consequently doses are kept as low
as possible, consistent with controlling symptoms.

Is there support?

Pemphigus Vulgaris Network

The Network is a support group for people living with Pemphigus
and Mucous Membrane Pemphigoid. The helpline offers information and
support to affected individuals, their families and carers, and
medical professionals.